Product datasheet
Anti-Alpha Skeletal Muscle Actin antibody ab113417
1 References 2 Images
Overview
Product name
Anti-Alpha Skeletal Muscle Actin antibody
Description
Rabbit polyclonal to Alpha Skeletal Muscle Actin
Specificity
ab113417 does not react to smooth muscle actin.
Tested applications
WB, IP, IHC-P
Species reactivity
Reacts with: Mouse, Rat, Chicken, Cow, Human
Immunogen
Synthetic peptide derived from N-terminus of Human skeletal muscle Actin. This sequence is
identical in Human, Rat, Mouse, Dog, Bovine, Guinea pig, Sheep and Frog origins.
Positive control
Mouse Skeletal Muscle lysate
Properties
Form
Liquid
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or 80°C. Avoid freeze / thaw cycle.
Storage buffer
pH: 7.20
Preservative: 0.01% Proclin
Constituents: 99% PBS, BSA
Purity
Immunogen affinity purified
Clonality
Polyclonal
Isotype
IgG
Applications
Our Abpromise guarantee covers the use of ab113417 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application
WB
Abreviews
Notes
Use a concentration of 0.1 - 1 µg/ml. Detects a band of approximately 42 kDa
(predicted molecular weight: 42 kDa).
IP
Use a concentration of 2 - 5 µg/ml.
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Application
Abreviews
IHC-P
Notes
Use a concentration of 2 - 5 µg/ml. Staining of formalin-fixed tissue requires
boiling tissue sections in 10 mM Citrate Buffer, pH 6.0 for 10 min followed by
cooling at RT for 20 min.
Target
Function
Actins are highly conserved proteins that are involved in various types of cell motility and are
ubiquitously expressed in all eukaryotic cells.
Involvement in disease
Defects in ACTA1 are the cause of nemaline myopathy type 3 (NEM3) [MIM:161800]. A form of
nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle
weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle
fibers on histologic examination. The phenotype at histological level is variable. Some patients
present areas devoid of oxidative activity containg (cores) within myofibers. Core lesions are
unstructured and poorly circumscribed.
Defects in ACTA1 are a cause of myopathy congenital with excess of thin myofilaments
(MPCETM) [MIM:161800]. A congenital muscular disorder characterized at histological level by
areas of sarcoplasm devoid of normal myofibrils and mitochondria, and replaced with dense
masses of thin filaments. Central cores, rods, ragged red fibers, and necrosis are absent.
Defects in ACTA1 are a cause of congenital myopathy with fiber-type disproportion (CFTD)
[MIM:255310]; also known as congenital fiber-type disproportion myopathy (CFTDM). CFTD is a
genetically heterogeneous disorder in which there is relative hypotrophy of type 1 muscle fibers
compared to type 2 fibers on skeletal muscle biopsy. However, these findings are not specific
and can be found in many different myopathic and neuropathic conditions.
Sequence similarities
Belongs to the actin family.
Cellular localization
Cytoplasm > cytoskeleton.
Anti-Alpha Skeletal Muscle Actin antibody images
Anti-Alpha Skeletal Muscle Actin antibody
(ab113417) at 1/500 dilution + Mouse
skeletal muscle lysate
Predicted band size : 42 kDa
Western blot - skeletal muscle Actin antibody
(ab113417)
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Skeletal muscle tissue stained with ab113417
at a dilution of 1/200.
Immunohistochemistry (Formalin/PFA-fixed
paraffin-embedded sections) - skeletal muscle
Actin antibody (ab113417)
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